{"id":23714,"date":"2023-04-17T04:16:00","date_gmt":"2023-04-16T20:16:00","guid":{"rendered":"http:\/\/csccm.org.cn\/?p=23714"},"modified":"2023-04-17T05:42:46","modified_gmt":"2023-04-16T21:42:46","slug":"nejm%e4%b8%b4%e5%ba%8a%e5%8c%bb%e5%ad%a6%e5%bd%b1%e5%83%8f%ef%bc%9aerdheim-chester%e7%97%85%e7%9a%84%e6%af%9b%e7%8a%b6%e8%82%be","status":"publish","type":"post","link":"https:\/\/csccm.org.cn\/?p=23714","title":{"rendered":"[NEJM\u4e34\u5e8a\u533b\u5b66\u5f71\u50cf]\uff1aErdheim-Chester\u75c5\u7684\u201c\u6bdb\u72b6\u80be\u201d"},"content":{"rendered":"\n<p><a href=\"https:\/\/www.nejm.org\/multimedia\/images-in-clinical-medicine\" class=\"\">IMAGES IN CLINICAL MEDICINE<\/a><\/p>\n\n\n\n<h1 class=\"wp-block-heading\">\u201cHairy Kidney\u201d in Erdheim\u2013Chester Disease<\/h1>\n\n\n\n<h3 class=\"wp-block-heading\">Nityasree Srialluri, Mohamed G. Atta<\/h3>\n\n\n\n<h3 class=\"wp-block-heading\">N Engl J Med 2023; 388:925<br \/>DOI: 10.1056\/NEJMicm2208776<\/h3>\n\n\n\n<figure class=\"wp-block-image size-large\"><img decoding=\"async\" src=\"https:\/\/www.nejm.org\/na101\/home\/literatum\/publisher\/mms\/journals\/content\/nejm\/2023\/nejm_2023.388.issue-10\/nejmicm2208776\/20230306\/images\/img_large\/nejmicm2208776_f1.jpeg\" alt=\"\"\/><\/figure>\n\n\n\n<p>A 62-year-old man undergoing abdominal ultrasonography for the evaluation of gallstones was found to have a retroperitoneal mass. A physical examination and the results of routine laboratory studies, including tests of renal function, were normal. A computed tomographic (CT) urogram showed soft-tissue infiltration of the kidneys (Panel A, arrows), severe hydronephrosis of both kidneys, and moderate splenomegaly. Subsequent positron-emission tomography\u2013CT showed no hypermetabolic activity. Stents were placed in both ureters, and a core-biopsy specimen of the perinephric soft tissue was obtained. Histopathological examination of the specimen revealed diffuse infiltration with pale-staining histiocytes (Panel B, arrows; hematoxylin and eosin staining) admixed with scattered lymphocytes and plasma cells. Immunostaining showed that the cells were strongly positive for CD68 and negative for S100 and CD1a. Molecular testing detected a\u00a0<em>BRAF<\/em>\u00a0V600E mutation. A diagnosis of Erdheim\u2013Chester disease was made. Erdheim\u2013Chester disease is a non\u2013Langerhans-cell histiocytosis that is typically manifested by sclerotic lesions in the long bones \u2014 a feature that this patient uniquely did not have. This disease is also associated with numerous extraosseous findings, including infiltration of the retroperitoneal perinephric tissue, which results in a \u201chairy kidney\u201d appearance on cross-sectional imaging, as was seen in this patient. Treatment with vemurafenib, a targeted inhibitor of the mutated\u00a0<em>BRAF<\/em>\u00a0V600E kinase, was started. At a 1-year follow-up visit, the patient had had no disease progression.<\/p>\n","protected":false},"excerpt":{"rendered":"<p>IMAGES IN CLINICAL MEDICINE \u201cHairy Kidney\u201d in Erdheim\u2013C [&hellip;]<\/p>\n","protected":false},"author":3,"featured_media":0,"comment_status":"closed","ping_status":"closed","sticky":false,"template":"","format":"standard","meta":[],"categories":[16,13],"tags":[],"_links":{"self":[{"href":"https:\/\/csccm.org.cn\/index.php?rest_route=\/wp\/v2\/posts\/23714"}],"collection":[{"href":"https:\/\/csccm.org.cn\/index.php?rest_route=\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/csccm.org.cn\/index.php?rest_route=\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/csccm.org.cn\/index.php?rest_route=\/wp\/v2\/users\/3"}],"replies":[{"embeddable":true,"href":"https:\/\/csccm.org.cn\/index.php?rest_route=%2Fwp%2Fv2%2Fcomments&post=23714"}],"version-history":[{"count":1,"href":"https:\/\/csccm.org.cn\/index.php?rest_route=\/wp\/v2\/posts\/23714\/revisions"}],"predecessor-version":[{"id":23715,"href":"https:\/\/csccm.org.cn\/index.php?rest_route=\/wp\/v2\/posts\/23714\/revisions\/23715"}],"wp:attachment":[{"href":"https:\/\/csccm.org.cn\/index.php?rest_route=%2Fwp%2Fv2%2Fmedia&parent=23714"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/csccm.org.cn\/index.php?rest_route=%2Fwp%2Fv2%2Fcategories&post=23714"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/csccm.org.cn\/index.php?rest_route=%2Fwp%2Fv2%2Ftags&post=23714"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}